The Daily Bruin, UCLA

UCLA Doctors Operate, Cure Rare Illness in Filipino Youth

MIRACLE: Overwhelming odds overcome in quest to save child from muscle-afflicting disease.

When doctors in the Philippines told Menchi Estares that nothing more could be done for her son’s life-threatening condition, she didn’t listen.

With the help of a nurse at the hospital where her son spent nearly half of his life, Menchi conducted her own search for a cure on the World Wide Web.

Her son, Paul Anthony Estares, was born with Prune Belly Syndrome, a rare condition where those afflicted lack stomach muscles and suffer from severe urinary tract dysfunction.

Paul, who recently turned one, suffered from serious constipation, breathing difficulty and damaging psychological ramifications due to the abnormal appearance of his stomach area.

Despite the doctors’ lack of faith, Menchi was determined to find help for her son.

She soon found the home page of the National Organization for Rare Disorders (NORD) and learned about Dr. Richard Ehrlich and Dr. Malcolm A. Lesavoy, who pioneered a revolutionary method for dealing with Prune Belly Syndrome in 1994.

“When I saw Dr. Ehrlich’s name, I felt something – hope,” Menchi said.

She soon wrote a letter to Dr. Richard Ehrlich at the UCLA Children’s Hospital, asking for his help.

“I asked him if he could save my son’s life,” she said.

Not only did Dr. Ehrlich agree to help Paul, but he also enlisted the support of a generous donor.

This mysterious donor, a close friend of Dr. Ehrlich’s and a past patient, has chosen to remain anonymous.

Having donated $50,000 to cover hospital fees and travel expenses for both Menchi and her child, this donor’s contribution cannot be ignored.

“I want to meet him,” said Menchi of the donor.

“There is no question in my mind he would have died,” without the surgery, Ehrlich said.

Ehrlic, along with a team of urologists and plastic surgeons, performed a three-part, 10-hour-long operation reconstructing Estares’ abdominal wall, his urinary tract and bringing down the testes.

“It was an honor to work with (the child),” said Lesavoy, the plastic surgeon on the team.

After the operation, “(Paul) cried when he saw me,” Menchi said.

Since this method was formulated in 1994, 35 to 40 patients have undergone the surgery and all have survived.

“If his problem was not fixed, it would have led to his demise,” Lesavoy said.

In most cases of the syndrome, the patient is born with a congenital problem, marked by distinct urological problems, such as a dilated kidney and bladder. In addition, the testicles are not fully descended and the abdominal wall lacks the proper muscles.

Since there are no muscles to hold the shape of the stomach, the belly protrudes and wrinkles abnormally, causing a prune-like appearance.

The most damaging ramification of this abnormal physical appearance is the psychological damage afflicted on the patient, according to Ehrlich and Lesavoy.

Most patients who suffer from this condition refuse to leave their homes or associate with others due to their appearance.

“After the procedure, the patient can function normally,” Lesavoy said.

“We are very fortunate that the mother was able to find us on the Internet.”

Paul is expected to fully recover and is scheduled to leave UCLA Children’s Hospital in the next week, after which he will return to the Philippines in less than two weeks.

“We never gave up hope,” Menchi said.

By Laura Luu
Excerpted from dailybruin.ucla.edu